Prolidase deficiency

Report of a second case with quantitation of the excessively excreted amino acids

Gerald F. Powell, Alexander Kurosky, Rose Mary Maniscalco

    Research output: Contribution to journalArticle

    21 Citations (Scopus)

    Abstract

    The second documented case of prolidase deficiency is presented. Clinical manifestations include chronic otitis media and sinusitis, dermatitis, and splenomegaly. Prolidase is undetectable in the white blood cells of the patient and near or less than the lower range of normal in each parent. The peptide chromatographic pattern of the urine is similar to that of the previously reported patient with prolidase deficiency. The quantity of amino acids excreted in urine per 24 hours is at least three times that of the upper range of normal (of these same amino acids) for the patients age group. More than 80% of the total amino acids excreted are in peptide form. The proline-to-hydroxyproline ratio suggests that the dipeptides are the catabolic products of other proteins in addition to collagen.

    Original languageEnglish (US)
    Pages (from-to)242-246
    Number of pages5
    JournalThe Journal of pediatrics
    Volume91
    Issue number2
    DOIs
    StatePublished - 1977

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    Prolidase Deficiency
    proline dipeptidase
    Amino Acids
    Reference Values
    Urine
    Peptides
    Dipeptides
    Sinusitis
    Hydroxyproline
    Splenomegaly
    Otitis Media
    Dermatitis
    Proline
    Leukocytes
    Collagen
    Age Groups
    Proteins

    ASJC Scopus subject areas

    • Pediatrics, Perinatology, and Child Health

    Cite this

    Prolidase deficiency : Report of a second case with quantitation of the excessively excreted amino acids. / Powell, Gerald F.; Kurosky, Alexander; Maniscalco, Rose Mary.

    In: The Journal of pediatrics, Vol. 91, No. 2, 1977, p. 242-246.

    Research output: Contribution to journalArticle

    Powell, Gerald F. ; Kurosky, Alexander ; Maniscalco, Rose Mary. / Prolidase deficiency : Report of a second case with quantitation of the excessively excreted amino acids. In: The Journal of pediatrics. 1977 ; Vol. 91, No. 2. pp. 242-246.
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    abstract = "The second documented case of prolidase deficiency is presented. Clinical manifestations include chronic otitis media and sinusitis, dermatitis, and splenomegaly. Prolidase is undetectable in the white blood cells of the patient and near or less than the lower range of normal in each parent. The peptide chromatographic pattern of the urine is similar to that of the previously reported patient with prolidase deficiency. The quantity of amino acids excreted in urine per 24 hours is at least three times that of the upper range of normal (of these same amino acids) for the patients age group. More than 80{\%} of the total amino acids excreted are in peptide form. The proline-to-hydroxyproline ratio suggests that the dipeptides are the catabolic products of other proteins in addition to collagen.",
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